NHS Sickle Cell and Thalassaemia Screening Programme Publications Launch Webinar Thursday 28th January 2021
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NHS Sickle Cell and Thalassaemia Screening
Programme
Publications Launch Webinar
Thursday 28th January 2021
Welcome to NHS Sickle Cell and Thalassaemia
Screening Programme’s Live Webinar
Thursday 28th January 2021
Chair: Professor Dame Elizabeth NAnionwu DBE CBE FRCN FQNI PHD
Emeritus Professor of Nursing, University of West London
Menti Questions
BACKGROUND
QUALITITES OF A GENETIC COUNSELLOR:
1. KNOWLEDGE OF THE GENETIC DISEASE
2. ABILITY TO PROVIDE & IMPART UP TO DATE INFORMATION
3. CAPACITY TO LISTEN AND BE EMPATHETIC
4. KNOWLEDGE OF MECHANISMS AVAILABLE TO SUPPPORT
INDIVIDUALS, FAMILIES & COMMUNITIES
5. ABILITY TO TAKE ACCOUNT OF SOCIO-CULTURAL DIVERSITY AND
RESPOND ACCORDING TO CLIENT’S NEED
6. ABILITY TO BE NON-DIRECTIVE AND NON JUDGMENTAL
7. ABILITY TO COPE WITH REPETITION
8. BE ACCOUNTABLE FOR ACTIONS AND OMISSIONS
9. MAINTAIN CONFIDENTIALITY
5 Counselling, Knowledge & Skills Guidelines - Background & introduction to the new Guidelines
KEY OBJECTIVE OF GENETIC COUNSELLING To provide the family with a realistic view of the situation, the nature of the inherited disorder already manifested in a family member, the risk of occurrence or re-occurrence, what this may mean in practical terms for all concerned, and to assist the family through what is often a difficult phase of their life… 6 Counselling, Knowledge & Skills Guidelines - Background & introduction to the new Guidelines
Aim & Objectives The aim of the competency is to update and provide practice guidance for those involved in providing genetic counselling services for those with and at- risk of sickle cell & thalassaemia. Practitioners include specialist nurses, antenatal & newborn screening coordinators / midwives, genetic counsellors and other relevant health and allied care professionals involved in providing the specialist counselling service Promote development of skills, knowledge and competence in order to meet the needs of the client group and enable formalised assessment of individuals. And, enhance the practitioner’s ability to meet the requirements of their professional code e.g. NMC 7 Counselling, Knowledge & Skills Guidelines - Background & introduction to the new Guidelines
Title: SCT Counselling knowledge and skills Core competencies: 1. Identification - Identify individuals and families who will benefit from testing and counselling 2. Communication - Understand the importance of effective communication in supporting individuals and families with, or at risk of having a baby with, sickle cell and thalassaemia 3. Supporting personal informed choice - Advocate for the rights of all individuals to make a personal informed choice 4. Knowledge and awareness - Understand the genetic basis and clinical implications of sickle cell and thalassaemia 8 Counselling, Knowledge & Skills Guidelines - Background & introduction to the new Guidelines
Title: SCT Counselling knowledge and skills 5. Use of genetic information, tests and results - Care and support individuals and their families prior to, during and following genetic testing 6. Maintaining SCT competence - Maintaining and updating SCT knowledge and skills through lifelong learning 7. Accessing information and resources - Obtaining and using information to support credible, current communication about sickle cell and thalassaemia 8. Ongoing support - Providing ongoing support to individuals and families with sickle cell and thalassaemia 9 Counselling, Knowledge & Skills Guidelines - Background & introduction to the new Guidelines
Additional
Learning Outcomes and practice indicators for assessment against
each competency
Formal assessment tool
Mapping to National Occupational Standards
UK Workforce National Occupational Standards for Genetics and
Genomics in Clinical Practice for non- genetics healthcare staff
Information on sources of further support and learning
10 Counselling, Knowledge & Skills Guidelines - Background & introduction to the new GuidelinesThank you
Counselling, Knowledge & Skills Guidelines - Background & introduction to the new Guidelines
11A Patients Perspective Laurel Brumant – Sickle Cell Society
Project delivery: Sickle cell and thalassaemia (SCT) counselling knowledge and skills Jamili Miah – Project and Implementation Lead NBS Screening Programme Empowering to deliver together
Organisations 14 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
Old SCT Counselling
Competences
A4 folder
Over 100 pages
Too much text / repetitive
Misleading title
Nowhere to capture
evidence of
accomplishment
Not easily accessible online
Not well implemented
15 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skillsUser needs • a shorter, concise set of competences that can be achieved within very busy workloads • an assessment record that shows trainee practitioners are working towards achieving the competences • signposting to education and training resources for trainee practitioners, especially those working in low prevalence areas where access to an expert clinical network is not as easy 16 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
Timeline
• First working group meeting
Sep 2018
• Chair - Dr Lola Oni
• Final working group meeting
• Piloted
March
2019 • Needed to be more user friendly
• Finalised
• Published
Sept 2019 • Blog
17 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skillsProject Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
1819 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
20 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
21 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
22 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
23 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
24 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
Feedback Resources are reviewed every 3 years Email your feedback/suggestions to PHE.screeninghelpdesk@nhs.net 25 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
Thank you 26 Project Delivery: Sickle cell and Thalassaemia (SCT) counselling knowledge and skills
Menti Questions
Jamili & LolaNHS Sickle Cell and Thalassaemia Screening Programme’s Live Webinar, 28th January 2021 Comfort Break
NHS Sickle Cell & Thalassaemia Screening Programme Publications go digital Cynthia Gill – SCT Programme Advisor Siobhan Ryan – SCT Project Lead
Digital by default
All the programme resources are now digital
•Transition to digital is supported by user research which show
that most women benefit from, and expect digital information
•We are supporting services in England to move to using
digital versions of the antenatal and newborn (ANNB)
screening and carrier leaflets
•Digital versions of the leaflets:
Are easy to edit, so they always contain the most up to date information
Are in a format that can be used with assistive technologies
Work well on smaller screens such as phones
Can be saved to a smart phone home screens like an app
Breast office managers meetingAccessible Information
Leaflets are required to meet Government accessibility
standards
The new format we are using:
•Makes the leaflets easy to find with search engines
•Can be accessed on a variety of devices
•Can be printed out by HCPs to be given to those who are not online
The language:
•Remains in “Plain English” – allowing the information to be easily
understood
•Jargon and abbreviations are avoided
•Technical terms are fully explained
Breast office managers meetingThe plan
Printed screening and carrier leaflets
are being phased out, with a move to
information which is digital by default
We have created a digital format for
•8 antenatal leaflets for adult carriers
•2 antenatal leaflets for women and couples
at risk of having a baby with a
haemoglobinopathy
•2 newborn leaflets for parents of babies
who carry a gene for unusual haemoglobin
And we blogged to inform professionals
in October 2020
https://phescreening.blog.gov.uk/2020/10/14/sickle-cell-
and-thalassaemia-carrier-leaflets-go-digital/
Breast office managers meetingThe process
Advises on content of Create standard leaflet
the leaflet and user
format and advise on
needs
programme requirements
Clinical Programme
advisor team
IEPP
Check for language &
accessibility and publish
to GOV.UK website
Breast office managers megAim of antenatal carrier leaflets To inform the pregnant woman of what her carrier status means to her and her baby Has information specific to her carrier status To invite baby’s biological father for screening Clear unambiguous language Links to other PHE publications
The result (1) Clear headings Standard layout Links in the text make navigation quick and easy Breast office managers meeting
The result (2) Graphics for healthcare professionals to use to explain inheritance to women or couples and discuss next steps and choices Breast office managers meeting
To consider
•Information is targeted to antenatal woman who is a
carrier. What about information for carrier fathers?
•Accessible carrier information for individuals who do not
read well?
•Accessible carrier information for individuals whose first
language is not English?
Breast office managers meetingLaboratory Handbooks Dr Yvonne Daniel
Handbooks • Update cycle – 2 years • Current versions published 2017 • Antenatal handbook • Approved • Waiting for final review • Newborn handbook • Pending final edits and approvals • Programme handbook • Pending final edits and approvals • Published as HTML 39 – Laboratory Handbooks
Handbooks
• Detailed guidance for the
laboratories:
• Methods
• Instruments
• Protocols
• Testing strategies
• Interpretation,
• Reporting
• Risk assessments
40 - Laboratory Handbooks• Incidents
• Queries
• Programme Helpline
Review criteria • Support Service
• Changes in practice
• New guidance
• Feedback from work shops/training events
• Advisory groups
• UK NEQAS
41 - Laboratory Handbooks2020 2021
What’s new: Newborn
• MSMS – guidance updated
• More users more cases more evidence
• Limitations of techniques
• More explicit in appropriate second test techniques
• Linkage with UKAS
42 - Laboratory Handbooks2020 2021
What’s new: Antenatal
• Language more consistent across handbooks
• Removed some schematics due to increasing complexity
• Emphasised co-inheritance of conditions
• Linkage with UKAS
43 - Laboratory HandbooksNHSE –
Genomics Laboratory Hubs
• 7 Genomics Centres
• 4 Haematology
• Implementation on going
• Ultimately no direct charge to requestor
• PHE engagement:
• Reporting
• Testing strategies for DNA transfused babies
• Ensure that it meets the needs of the screening programme
44 - Laboratory HandbooksThank you 45 - Laboratory Handbooks
Menti Questions Siobhan, Cynthia and Yvonne
Two New Publications and the Consultation with Sickle Cell Service Users Iyamide Thomas, NHS Engagement Lead, Sickle Cell Society
Background & Objectives • In 2018 NHS SCT Programme Commissioned Sickle Cell Society and UKTS to work collaboratively with them in ‘Engagement, Outreach and Programme Development’ Project. Work Specification included: • Review and update publication ‘Sickle cell disease in childhood: standards and guidelines for clinical care’ (2010 Edition) –’Paediatric Standards’ • Project Advisory Group decided that this publication should remain focused on the clinical management of sickle cell so added: • Update of ‘Parent’s Guide to Managing Sickle Cell Disease’ to include the wider determinants of health relevant to living with sickle cell (2012 Edition) – ‘Parents Handbook’ Two New Publications and the Consultation with Sickle Cell and Service Users
Updating Two New Publications
‘Paediatric Standards’ ‘Parents Handbook’
Two New Publications and the Consultation with Sickle Cell and Service UsersConsultation Outputs Consult stakeholders (i.e. sickle cell service users) to solicit opinion on any changes they might want to see and whether the Parents Handbook (PH) should be reproduced in print or electronic format. Design and pilot user questionnaire at support group (PH only) Finalise questionnaires for hard copy and online consultation Disseminate hard copy to parents in Brent and Milton Keynes SCaT for on site completion (PH only) Conduct service user focus group in central London Design & Disseminate advertising graphics for online questionnaires Analyse questionnaires and produce report for Editorial Teams of both publications to incorporate user feedback as necessary Two New Publications and the Consultation with Sickle Cell and Service Users
Consulting Parents Face-to-Face
Parents’ Support Group Parents Focus Group in Euston
Two New Publications and the Consultation with Sickle Cell and Service UsersGalvanising Online User Input Two New Publications and the Consultation with Sickle Cell and Service Users
Galvanising Online User Input Two New Publications and the Consultation with Sickle Cell and Service Users
Some Summary Results for Parents Handbook
51 completed questionnaires returned – half done online
20% respondents knew nothing about SCD before the PH
97% stated they found the 2012 edition useful for reasons such as:
“Gives different signs and symptoms of the disease and what to look out for”
Parents who did not find the book useful said:
“Sometimes have difficulty reading the English or medical jargon”
90% felt a printed copy of the PH should be given to all new parents
67% said child’s symptoms less manageable without book
What format of the PH will you find most useful?
65% (Both internet and hard copy) 33% (Hard copy) 2% (internet copy)
What new information would you like in the Handbook?
“Parents’ Experiences”, “Breaking news of SCD to child”, “Direct references
to legal /statutory support for parents to access when making bids to
employers for flexible working” “ Extra curricular activities like ballet” “More
information on potential need for counselling psychological support,
particularly in adolescence and onwards”
Two New Publications and the Consultation with Sickle Cell and Service UsersParents’ Handbook – What was Said
“I personally have learnt so much from
this book. It has been like our second
doctor in my house”
“The book is like the sickle cell bible,
very useful”
Two New Publications and the Consultation with Sickle Cell and Service UsersPaediatric Standards – Updated!
Second Edition - 2010 Third Edition - 2019
Two New Publications and the Consultation with Sickle Cell and Service UsersParents Handbook – Updated!
Third Edition - 2012 Fourth Edition - 2021
Two New Publications and the Consultation with Sickle Cell and Service UsersSickle Cell Disease in Childhood: Standards and Recommendations for Clinical Care. Dr Moira Dick and Professor David Rees on behalf of PHE and the Sickle Cell Society
httpstandards/
https://www.sicklecellsociety.org/paediatricstandards/
Dr Moira Dick and Professor David Rees on behalf of PHE and the
Sickle Cell SocietySickle cell disease as it was then
• a rare childhood disease in 1970s- 90s
• considered an acute hospital issue
• childhood mortality 10% due to infection, stroke, acute
splenic sequestration, worsening anaemia, acute chest
syndrome
• approximately 2000 adults and children in the UK with
main populations in London and Birmingham (survey
1979) Davis LR et al BMJ 1981 282
• antenatal and newborn screening was inconsistent
across country
• Infants not always followed up after screening Assessment of
care of children with sickle cell disease: implications for neonatal screening programmes. R I Milne
BMJ 1990 300Implications due to lack of newborn screening and quality of follow up • infant deaths from pneumococcal sepsis or acute splenic sequestration occurred before diagnosis made • understanding of cerebral vasculopathy limited as no MRI/TCD • most paediatricians had little or no training or experience in sickle cell disease • was it a paediatric or a haematological condition? • resources and services varied enormously across the country • widespread experience of stigma • racist attitudes prevalent MRI magnetic resonance imaging TCD transcranial Doppler scanning
First edition paediatric standards 2005
• developed in 2005 on behalf of BSH to support roll out
of universal newborn screening and aimed at those
clinicians working in areas of low prevalence
• dealt with organisation of care as much as clinical
recommendations
• audit standards were included – timeliness into care
and prescription of penicillin, timeliness of Pneumovax,
follow up and failsafe arrangements, coverage of TCDs
• evidence only in four areas- penicillin prophylaxis,
TCD screening , hydroxyurea and incentive spirometry
• mainly based on good practice from UK and USA
BSH : British Society of Haematology2nd edition 2010 Sickle cell disease in childhood Standards and Guidelines for clinical care • added a standard on data collection and National Haemoglobin Registry (NHR) • included clinical peer review quality requirements and specialist services definitions
Latest edition 2019 Standards and clinical recommendations for care of children with SCD • recommendations on cerebrovascular disease, preoperative transfusion and hydroxycarbamide therapy updated as evidence now available in these areas • standards strengthened in line with Public Health England guidance and Metric Definition Sets that inform the Specialised Services Quality Dashboard commissioned by NHS England • new standards on coverage of children prescribed hydroxyurea, coverage of children on NHR and completion of annual review • a new information technology system linked with NHR for referring infants from newborn screening into treatment introduced. https://www.gov.uk/government/publications/sickle-cell-and-thalassaemia-screening-newborn-outcomes-system/clinical- user-guide-newborn-outcomes-system
Grade A recommendations
Requires at least one clinical trial
• the TWiTCH study looked at the safety of switching children with
abnormal TCDs from regular transfusions to hydroxycarbamide and
found that hydroxycarbamide was equivalent to transfusion, with no
increase in TCD velocities or cerebrovascular events (A)
• children with HbSS and HbS/β0 thalassaemia undergoing low- and
moderate-risk surgery should have a preoperative transfusion to
increase the Hb level to 100 g/L (A)
DeBaun MR, Gordon M, McKinstry RC, et al. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med 2014
Aug 21;371: 669–710
WareRE, DavisBR, SchultzWH, et al. Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in
children with sickle cell anaemia—TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-
inferiority trial. Lancet 2016; 387: 661–70.
Howard J, Malfroy M, Llewelyn C et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised,
controlled, multicentre clinical trial. Lancet 2013; 381: 930–8.Hydroxycarbamide ( hydroxyurea) • hydroxycarbamide should be offered to all children with HbSS/Sβ0 thalassaemia aged 9–42 months regardless of the clinical severity of their illness. (A) See Standard 6. • hydroxycarbamide should be offered to all children older than 42 months who have recurrent episodes of acute pain or who have had two or more episodes of acute sickle chest syndrome (A) • hydroxycarbamide should be offered to all children older than 42 months whose lives are significantly affected by symptoms of SCD, including those with frequent episodes of pain that disrupt normal activities (A) • hydroxycarbamide should be offered to all children older than 42 months who are at high risk of progressive organ damage caused by SCD, including those with hypoxemia, significant albuminuria, conditional TCD velocities, or significant anaemia (steady state Hb
Standards- alignment and data collection • Standard 1 (SCT-S08): Sickle cell and thalassaemia screening – reporting newborn screen-positive results to parents Newborn clinical outcome system (NBO) • Standard 2 (SCT-09 HAEM 4A): Sickle cell and thalassaemia screening – timely follow up, diagnosis and treatment of newborn infants with a positive screening result NBO • Standard 3: (HAEM 4B) Timeliness of penicillin prophylaxis NBO • Standard 4: Coverage of pneumococcal immunisation at 2 years ? NHR • Standard 5 (HAEM02) Coverage of transcranial Doppler (TCD) scanning ? NHR • Standard 6: Coverage of hydroxycarbamide (hydroxyurea) therapy ? NHR • Standard 7: Coverage of children identified through the screening programmed subsequently registered on the national haemoglobinopathy registry NBO/NHR • Standard 8: ( HAEM08) Coverage of children who have had an annual review NHR https://www.gov.uk/government/publications/sickle-cell-and-thalassaemia- screening-newborn-outcomes-system/clinical-user-guide-newborn-outcomes- system
Acknowledgements – and thanks to
everyone who took part in the consultation
Writing group members:
• Dr Kofi Anie,MBE Consultant Clinical Psychologist, Brent Sickle Cell and Thalassaemia Centre, London
• Dr Subarna Chakravorty, Consultant Paediatric Haematologist, King’s College Hospital, London and member of the
peer review team
• Susan Raybould, Specialist Nurse Haemoglobinopathy, Birmingham Sickle Cell and Thalassaemia Service
• Cathy Coppinger, NHS Sickle Cell & Thalassaemia Screening Programme, Public Health England
• Giselle Padmore-Payne, Senior Clinical Nurse Specialist Haemoglobinopathy Transition, King’s College Hospital,
London
• Iyamide Thomas, Sickle cell Society
• Dr Jacky Wilson, Medical Editor
and additional specialist input from:
• Dr Karl Atkin, Department of Health Sciences, University of York
• Dr Mark Velangi, Consultant Paediatric Haematologist, Birmingham Children’s Hospital and member of the peer
review team
• Dr Jo Howard, Consultant Haematologist, Guy’s Hospital, London and chair of the UK Forum on Haemoglobin
Disorders ( until 2019)
• Dr Kate Ryan, Consultant Haematologist, Manchester Royal Infirmary and chair of the Clinical Reference Group for
Haemoglobinopathy Specialist Commissioning.(until 2019)Menti Questions
Iyamide & MoiraThank you and closing remarks Chair: Professor Dame Elizabeth N Anionwu DBE CBE FRCN FQNI PHD Emeritus Professor of Nursing, University of West London
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